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2015

International Archives of Medicine

http://journals.imed.pub

Section: Gastroenterology
ISSN: 1755-7682

Vol. 8 No. 21
doi: 10.3823/1620

Chronic Inflammatory Polyneuropathy Associated


With Ulcerative Colitis: A Reported Case In Literature
case report

abstract
Background: Peripheral neuropathy is known to be related to inflammatory bowel disease and it is one of the most frequently reported neurologic complications. Various studies have found peripheral
nervous system complications, rather than central nervous system
involvement, to be predominant. In the literature, there are a few cases of inflammatory polyneuropathy developed in the course of ulcerative colitis: 5 cases of Guillain-Barr syndrome, one case of chronic
inflammatory demyelinating polyneuropathy, 10 cases of neuropathy
and one case of perineuritis.

Case presentation: We describe a case of chronic inflammatory


polyneuropathy associated with ulcerative colitis.

Conclusion: Peripheral neuropathy is not a common manifestation of inflammatory bowel disease, highlighting the need for careful
exclusion of other causes of neuropathy when both conditions are
encountered in clinical practice.
Keywords
Chronic inflammatory demyelinating polyneuropathy, Guillain-Barr syndrome,
Nerve biopsy, Ulcerative colitis, inflammatory bowel disease

Background
Ulcerative colitis (UC) is a chronic relapsing inflammatory bowel disease (IBD) showing immunologic abnormalities and associated with
autoimmune states. It affects individuals throughout their life. Extraintestinal manifestations of UC are well documented, but their association with neurological involvement is rare [1-8].
There are a few reports of neurologic involvement in this setting
without previous medication. The occurrence of perineuritis associa-

Under License of Creative Commons Attribution 3.0 License

Maria Sheila Guimares


Rocha1,
Claude Vital3,4,
Alain Lagueny3,4,
Luiz Carlos de Abreu2,
Alzira Alves de Siqueira
Carvalho2
1 Neurology Department, Santa
Marcelina Hospital, So Paulo, Brazil.
2Neuroscience Department, ABC Medical
School, Santo Andr, Brazil, and
Study design Laboratory and Scientific
Writing, Department of Community
Health, ABC Medical School. Brazil.
3 Neurology Department, EA 2966
Neurobiology of Myelin Disorders.
4 Neurophysiology Department,
CHU Bordeaux, Hpital Pellegrin,
France Bordeaux, France.

Contact information:
Alzira Alves de Siqueira Carvalho.
Neuroscience Department, ABC Medical
School, Santo Andr, Brazil and Study
design Laboratory and Scientific Writing,
Department of Community Health, ABC
Medical School. Brazil

alzira@myogenetica.com.br

This article is available at: www.intarchmed.com and www.medbrary.com

2015

International Archives of Medicine

Section: Gastroenterology
ISSN: 1755-7682

ted with UC has been reported in only one case by


Chad et al. [2].

Case presentation
A 62-year-old caucasian woman was referred to
the Hospital complaining of dorsal thoracic pain,
muscular weakness and hypoesthesia of her legs
for 4 months. In the last fifteen days she gets worsening of weakness and had become quadriparetic. Examination revealed a marked weakness of
both legs and mild weakness of her arms (MRC
grade 2 in lower limbs and grade 4 in upper limbs).
Muscle tone was decreased and tendon reflexes
were absent in the legs and decreased in her arms.
Sensation of light touch was impaired in a glove
and stocking type distribution in lower and upper
limbs.
Vibration and position sensations were diminished in the legs. There was no sensory level to any
modality. The cranial nerve functions were normal.
The biochemical profile, liver functions, vitamin B12,
folic acid, and thyroid hormones were normal. Serological tests for Epstein-Barr virus, cytomegalovirus,

Vol. 8 No. 21
doi: 10.3823/1620

hepatitis B and C virus, and human immunodeficiency virus were all negative. The ESR was 75 mm/
hour.
Collagen diseases and malignancy were ruled
out by appropriate laboratory tests. Serum protein
electrophoresis showed a mild increase of gammaglobulin. The CSF protein was 119 mg/dl (normal:
10-40 mg/dl). The myelogram and image studies
(cervical and lombar MRI) were normal.
The conduction studies are shown in Table 1.
Needle examination showed neurogenic patterns
in both legs.
Neuromuscular biopsy revealed a inflammatory
mononuclear cell infiltration in endoneurium with
perivascular infiltrates without capillary wall modification and isolated inflammatory cells scattered
also in endoneurium and moderate inflammatory
infiltration of lymphocytes and monocytes in perivascular space also without inflammation in vessel
walls of muscle (Fig. 1, 2, 3).
The patient was treated with IVG, 400 mg/kg/
day, five days, with no improvement of symptoms.
Two weeks afterwards she developed intense rec-

Table 1. Conduction studies results of our patient.


MNCV (m/s)

CMAP amplitude(mV)

DL (ms)

SNVC (m/s)

SNAP amplitude(V)

R median

46.4

3.4

4.0

ND

ND

L median

44.8

6.2

3.8

51.

2.9

R ulnar

53.0

7.6

3.4

ND

L ulnar

50.0

8.6

2.9

48.2

R peroneal

31.0

1.9

4.7

L peroneal

29.1

0.8

5.6

R tibial

34.9

4.6

5.3

L tibial

37.0

4.0

4.1

R sural

ND

L sural

ND

Nerves

ND: not determinable; average(SD); R: right; L: left; Control values: MNCV, median= 55(4.5), DL= 3..8(0.4),
CMAP amplitude= 12(2.4); ulnar= 54,6(3.6), DL= 2,6(0,3), CMAP amplitude= 9..5(2..5); peroneal= 49(5..5), DL= 4..5(0.6),
CMAP amplitude= 6.,3(1.6); tibial= 52.6, DL= 3.0, CMAP amplitude= 24; SNCV, median= 52(3.,5);
SNAP amplitude= 12.5(3.4) ; SNAP amplitude= 5.5 ; sural= 48(4), SNAP amplitude= 16(4..5).

This article is available at: www.intarchmed.com and www.medbrary.com

International Archives of Medicine

Section: Gastroenterology
ISSN: 1755-7682

2015
Vol. 8 No. 21
doi: 10.3823/1620

Figure 1. H
 &E stain. Nerve: Perivascular lymphocyte inflammation in endoneurium.

Figure 2. H
 &E stain. Nerve: Perivascular lymphocyte inflammation in perineurium.

Figure 3. H
 &E stain. Muscle: Perivascular lymphocyte inflammation in perimysium.

Figure 4. H
 &E stain. Lymphocytic infiltration of
the intestinal mucosa and architectural
distortion of the crypts.

tal bleeding with feelings of weakness, dizziness,


faintness and diarrhoea. The colonoscopy suggested UC and colon biopsy confirmed the diagnosis
(Fig. 4). Therapy with steroid enemas and sulfasalazine, resulted in partial response. A sigmoidectomy
was performed because the sigmoid colon was the
most affected by colitis, which brought the patient to her normal control of intestinal symptoms.
A pulse of methylprednisolone resulted in a pro Under License of Creative Commons Attribution 3.0 License

gressive improvement of neurological symptoms,


making ambulation possible again. Azathioprine 2
mg/kg daily was added and patients neurological
condition continued to improve for 3 years and
after that the patient never returned.

2015

International Archives of Medicine

Section: Gastroenterology
ISSN: 1755-7682

Discussion
Peripheral neuropathy (PN) is known to be related
to IBD and it is one of the most frequently reported neurologic complications. Various studies have
found peripheral nervous system complications,
rather than central nervous system involvement, to
be predominant. The reported frequency of PN in
IBD will vary greatly among published studies, with
estimates ranging from 0% to 39% due to selection
bias, use of different definitions of the disease, or
population characteristics [9].
Ulcerative colitis (UC) is a chronic relapsing inflammatory bowel disease with various extra-intestinal
manifestations and there are a few reports of neurologic involvement in this setting without previous
medication (Table 2). In 1985, 2 elderly patients
with Guillain-Barr syndrome (GBS) were reported
while the UC was in remission [1]. In 1989, a case
of chronic polyneuropathy was reported associated
with UC [3]. In the same year, in a series of 60 cases
of CIDP, Barohn et al briefly mentioned one patient
who also had inflammatory bowel disease [10].
Lossos et al. reported a patient (case 3) with acute demyelinating polyradiculoneuropathy 12 years
after the UC had started [4]. A case of GBS was

Vol. 8 No. 21
doi: 10.3823/1620

described in a patient with the diagnosis of UC 30


years earlier [5]. A congenital GBS associated with
maternal UC was reported by Bamford et al. [11].
A 6-year-old girl developed an axonal sensorimotor
polyneuropathy a few days before intestinal symptoms with bloody diarrhea and abdominal pain.
Colon biopsy established the diagnosis of ulcerative colitis [6]. In 2005, Gondin et al presented the
largest case series of PN in patients with IBD. Both
demyelinating (30%) and non-demyelinating neuropathies were observed. Eighteen patients with CD
and 15 patients with UC were identified after other
PN causes were excluded [12].
A case reported by Yesilova et al described an
axonal motor polyneuropathy in a patient with ulcerative colitis. Symptoms of neuropathy occurred
during active colitis [8].
In 2007, Zezos et al reported a case of acute motor and sensory neuropathy during a flare of ulcerative colitis in a 28-year-old male despite treatment
with mesalazine enemas and suppositories simultaneously with rapidly deteriorating weakness and
needle sensation in both legs [7].
In 2010, a 59-year-old male patient developed
GBS during relapse of UC [13].

Table 2. Characteristics of 30 patients with UC who developed peripheral neuropathy.


year

gender

age

Num cases

type

Zimmerman et al

1985

59

GBS

Chad et al

1986

50

PN

Konayaga et al

1989

57

PN

Roca et al

1999

69

GBS

Bamford et al

2002

NO

34w

GBS

Greco et al

2004

PN

Gondin et al

2005

11M/4F

24-72

15

PN

Yesilova et al

2006

47

PN

Zezos et al

2007

28

PN

Kristallis et al

2010

59

GBS

Figueroa et al

2013

3M/1F

51-85

RPN/PN

Author

PN: peripheral neuropathy, GBS: Guillain-Barr syndrome, RPN: radiculoplexus neuropathy, NO:not obtained

This article is available at: www.intarchmed.com and www.medbrary.com

International Archives of Medicine

Section: Gastroenterology
ISSN: 1755-7682

A population-based cohort study demonstrated


772 patients diagnosed with IBD(UC and Crohn
disease(CD)). Only nine patients, aged 34 to 86
years developed peripheral neuropathy. Six out 9,
with UC and 3 with CD. The neuropathies comprised two phenotypes: radiculoneuropathy and chronic sensorimotor distal polyneuropathy. However, 3
patients were diabetic [14]. The clinical and electrophysiologic features of our patient were compatible
with axonal polyneuropathy with MNCV abnormalities suggesting additional demyelinating features.
The occurrence of perineuritis associated with
UC has been reported in only one case by Chad
et al. [2]. Their patient had a chronic polyneuropathy and the CSF protein content was 875 mg/
dl. He showed a gradual improvement with steroid
therapy.
King emphasized that the cellular infiltration is
more conspicuous in the epineurium in vasculitis
whereas there is an infiltration in the endoneurium
in autoimmune diseases [15].
Our patient showed only cellular infiltration in
endoneurium and the diseases most commonly
associated with vasculitis in endoneurium were
excluded. All the cases cited above did not mentioned about nerve biopsy. Also, they did not
specified the location of involvement by inflammatory cells except the paper published by Chad
et al [2].
The pathogenesis of UC remains unclear but
evidence suggests that an auto-immune antibodymediated mechanism is involved.

2015
Vol. 8 No. 21
doi: 10.3823/1620

Conclusion
Taken together, the data suggest that the neuropathy reported in our patient is inflammatory probably due to an autoimmune-mediated mechanism suggesting that both diseases had a common
pathogenesis.
The findings reported above show that PN in IBD
is not a common manifestation of IBD, highlighting
the need for careful exclusion of other causes of
neuropathy when both conditions are encountered
in clinical practice.

Consent
Written informed consent was obtained from the
patient for publication of this case report and any
accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Competing interests
The authors declare that they have no competing
interests.

Authors contributions
All authors participated in results collection, study
design and manuscript draft. All authors agreed
with the final version of the manuscript.

Acknowledgements
The authors state that the study received financial
support from Foundation of Support to Research
of Sao Paulo State (FAPESP) and Neuroscience Department of ABC Medical School.

Under License of Creative Commons Attribution 3.0 License

International Archives of Medicine

Section: Gastroenterology
ISSN: 1755-7682

2015
Vol. 8 No. 21
doi: 10.3823/1620

References
1. Zimmerman J, Steiner I, Gavish D, Argov Z. Guillain-Barr
Syndrome: a possible extraintestinal manifestation of ulcerative
colitis. J Clin Gastroenterol 1985, 7: 301-303.
2. Chad DA, Smith TW, DeGirolami U, Hammer K. Perineuritis and
ulcerative colitis. Neurology 1986, 36: 1377-1379.
3. Konagaya Y, Konagaya M, Takayanagi T. Chronic polyneuropathy
and ulcerative colitis. Jpn J Med 1989, 28: 72-74.
4. Lossos A, River Y, Eliakim A, Steiner I. Neurologic aspects of
inflammatory bowel disease. Neurology 1985, 45: 416-421.
5. Roca B, Moreno I, Meneu E. Ulcerative colitis and acquired
demyelinating neuropathy (Guillain-Barr syndrome). Neth J
Med 1999, 54: 129-130.
6. Greco F, Pavone P, Falsaperla R, Sorge G.J. Peripheral neuropathy
as first sign of ulcerative colitis in a child. Clin Gastroenterol.
2004, Feb; 38(2): 115-7.
7. Yeilova Z, Naharci I, Uygun A, Ula HU, Daalp K. Motor axonal
polyneuropathy in the course of ulcerative colitis: a case report.
Turk J Gastroenterol. 2006 Mar; 17(1): 58-61.
8. Zezos P, Mpoumponaris A, Koutsopetras P, Vounotrypidis P,
Molyvas E, Vadikolias K, Moschos I, Kouklakis. Acute motor
sensory polyneuropathy (AMSAN) complicating active ulcerative
colitis with a patchy distribution. G.Acta Gastroenterol Belg.
2007 Apr-Jun; 70(2): 226-30.
9. Moris G. Inflammatory bowel disease: an increased risk factor
for neurologic complications.World J Gastroenterol 2014, Feb 7;
20(5): 1228-37.
10. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic
inflammatory demyelinating polyradiculoneuropathy : clinical
characteristics, course, and recommendations for diagnostic
criteria. Arch Neurol 1989, 46: 878-884.
11. King King RHM. Atlas of Peripheral Nerve Pathology. London:
Arnold; 1999: 1217
12. F. A. A. Gondim, T. H. Brannagan, H. W. Sander, R. L. Chin,
N. Latov. Peripheral neuropathy in patients with inflammatory
bowel disease. Brain. 2005 Apr; 128 (Pt 4): 867-79.
13. Krystallis CS, Kamberoglou DK, Cheilakos GB, Maltezou MN,
Tzias VD. Guillain-Barr syndrome during a relapse of ulcerative
colitis: a case report. Inflamm Bowel Dis 2010 Apr; 16(4): 555-6.
14. Figueroa JJ, Loftus EV Jr, Harmsen WS, Dyck PJ, Klein CJ.
Peripheral neuropathy incidence in inflammatory bowel disease:
a population-based study. Neurology. 2013, Apr 30; 80(18):
1693-7.
15. King RHM. Atlas of peripheral nerve pathology, 1999 Arnold,
London, pp 1-217.

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